a .-lrl .11

3'_i

S .-ll CIAl . Ant 11'

'rahte a .

sr

Uncommon Unto .In Which

Adnlt SWei,A

k Efccptoo (

,I

. m

n IdI nt,r,l ltoo

Cur to :nn lnc m slilmt clu e~, I'nlmunan ran Ncnu,i, Cnis ,

~rnl 111 ,n+old malr.

non . . .o,,

dLtr

anon tram

iaruhle-oedcr 1155 wmraty Aonopulmonur • wmJax Tron s :uierin,a• f rRxn116alar IIC rl Cnnrplem Irnnsnmirinn rte great unene. ssdh *ernrieular stir l aefeu Tnucey d leech .vnrI, ~mml, viii h-srcJrdia

Tma1 arnInkIo pnlmnror, no • u,nn¢Irun is=emnn ,r.. aim

JTCC Vol. Is. No 2 Acar,l IWfr311-42

SV ( VAI . 1'M1tr f .Ki f:

Terr, ncr nl t

k,r with oh ,enl

important risk is infective etdocarditis, whereas beginning in the 3rd decade, heart failure is the major complication in patients with a sizable left to right shunt . Adult survival with a nonrestrictive patent ductus artenosus is more likely to occur with suprasystemic pulmonary vascular resistance and reversed shunt . Anomalous course of coronary arteries . Widespread use of coronary arteriography together with careful pathologic studies have disclosed the ectopic origins and anomalous course of the extramural coronary arteries- The proximal course of the anomalous coronary artery is ehnically more important than an ectopic aortic origin . The greatest risk occurs when the left coronary artery arises from the right aortic sinus and passes between the aorta and right ventricular outflow tract, a disorder mitre common in males . The

anomaly is generally not recognized until early to mid,aduli life . when it announces itself by angina pectoris . myocardial infarction or sodden death, especially during or immediately after exercise. Ventricular septal defect . Adult survivors generally fall info two groups : I) those whose defects have either closed spontaneously to have remained small or have become small and clinically inapparent . and 2) those who have nunrestnclive defects with pulmonary vascular disease and reversed shunt . Adult survival with a moderately restrictive defect and significant left to right shunt is exceptional . Longevity is idfected by cvexisiing aortic regurgitation or infective endocanditis . TetralngyofFallot . Eleven percent offiatients, are alive at 20 years . b7e at 30 years and 3% at 40 years . In patients with tetralogy of Fallot and pulmonary atresia . pulmonary blood flow is maintained by aortic to pulmonary collateral vessels. Systemic hypertension is a special concern because increased afterload is imposed on both the right and left ventricles, with what appears to be more dire consequences on the right ventricle . Acquired calcific aortic stenosis produces similar effects . Infective endacarditis an an incompetent aortic valve can result in acute severe regurgitation into both ventricles . with catastrophic consequences .

References I . Child r5, 1'eelntt 1K . trials sarlival patterw : a Perloff JK, Chkld 75 . eds. Csngrritel Heart Disease pnia wit Snnnders .1991.

narrawig hate. In: in Adults. Phdedet-

Cyanotic Congenital Heart Disease : Hematologic Management MARY C, TERRITO . MD, MICHAEL H . ROSOVE . MD

Regulatlm of erythrocyte muss . Hema ologic problems ire common and important in adult patients with cyanotic congenital heart disease and play a significant role in the clinical course of these disordss . En7hruc'xre mass is regulated chiefly by factors that affect lissue oxygenation . In response to tissue hypoxia . erythropoiettc is produced by specialized sensor cells in the kidneys . Erylhnopoietin is a major unmoral factor that induces proliferation and differen. tiation of erythroid cells in the bone marrow and in turn results in an increased number of red blood cells released iota the circulation and an increase in the erythrocyte mass. When congenital beast disease is associated with hypoxcmia . etythropuietin levels merriest and secondary esythrocytosis ensues. Tile increased erythrocyte mass may resolve the deficit in tissue oxygenation and establish a new equilib-

rium at a higher hematocnt, but an excessive increase in erythrocyte mass can impair lissue oxygen delivery because of inegcased blood viscosity. Iron deficiency also significantly affects blood viscosity It). in contrast to normal biconcave erythrocytes . iron-deficient erythrocytes are relatively rigid microspherocytes, resisting deformation in the microcirculation- thus increasing whole blood viscosity . Treatment of compensated versus decompensated erythrocytotis. Cyanotic patients have been classified into two groups those with "compensated" and those with " decompensated" erythrocytosis (2). Patients with compensated erythrocytesis establish equilibrium hematacrits in an ironreplete state, wild absent or mild symptoms attributable to hyperviscosiry . Hematocrit levels are occasionally o70%. Phlebotomy is seldom required in these individuals and .



IArr Is" . Ix, N.,-= )oossi 1Oti 11 1- 4 ,

TEHRITO ANn HosovF ('YANGrl(' (TiSOII51TA: .HEART DISEASE' HEMATOLOGIC MANAGEMENT

if inappropriately employed . results in symptomatic iron deficiency and an increase in whole blood viscosity Imicrospherocyres), Patients with decnmpensated crythracytosis exhibit urotzbte increasing hemalocrit levels and recurrent symptoms attributable to sluggish tissue perfusion . Symploms related to hppcrvinn lily include headache . fniipuei faintness, dizziness, visual disturbance, paresthesia, irritahility, myalgia . reduced mentalien and anorexia- Each symptom should be graded as mild, moderate. tnarked (it severe to provide the basis for phlebotomy 13) . Cerehrovaccular accidents . These sometimes occur in cyanotic infants and young children . especially those with iron deficiency - In cyanotic adult patients, however, erytilrocytosis itself does not appear to he a significant risk faclor far stroke . even when the hemalocrit level is >655t and even in the decnmpensated setting (3) . The relation hetween elevated hemalocrit levels and cercbral blood flow is an important and incompletely' resolved queetien t4-ht- bat phlebotomy is nor advised if the objective is to reduce the perceived risk of stroke. Phlebotomy is recommended only in patients with ivntpamnarrc hyperviscosily when hemalocrit levels exceed 65Sf, provided dehydration is not the cause . Dehydralion can result in a rapid increase in the h .matoCrit level . provoking hypcrviscosity symptoms . Treatment is volume replacement . not phlebotomy . Phlebotomy, rnlume replacement and iron replacement . Symptoms of iron deficiency can be indistineuishahlr from hyperviscosity . but symptomatic hyperviscosity rarely occurs in iron-replete cyanotic patients with hemalocrit levels

Cyanotic congenital heart disease: hematologic management.

a .-lrl .11 3'_i S .-ll CIAl . Ant 11' 'rahte a . sr Uncommon Unto .In Which Adnlt SWei,A k Efccptoo ( ,I . m n IdI nt,r,l ltoo Cur to :nn...
188KB Sizes 0 Downloads 0 Views